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Von Willebrand disease

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https://www.readbyqxmd.com/read/29665194/replacement-therapy-during-surgery-in-von-willebrand-disease-needs-personalization
#1
E Berntorp
No abstract text is available yet for this article.
April 17, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29651351/acquired-von-willebrand-disease-associated-with-mantle-cell-lymphoma
#2
Dominique Maas, Britta Laros-van Gorkom, Sanne Gianotten, Marjan Cruijsen, Waander van Heerde, Marten Nijziel
We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a mantle cell lymphoma. A 61-year-old male suffered from recurrent bleeding symptoms since a few months. Initially, physical examination was normal. von Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were low (0.31 IU/ml and 0.43 IU/ml, resp.). Ristocetin cofactor activity (VWF:RCo) was 0.09 IU/ml, and collagen binding activity (VWF:CB) was 0.10 IU/ml. VWF:RCo/VWF:Ag ratio was 0.29, and RIPA value was normal...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29626372/bleeding-related-hospitalization-in-patients-with-von-willebrand-disease-and-the-impact-of-prophylaxis-results-from-national-registers-in-sweden-compared-with-normal-controls-and-participants-in-the-von-willebrand-disease-prophylaxis-network
#3
E Holm, K Steen Carlsson, S Lövdahl, A E Lail, T C Abshire, E Berntorp
INTRODUCTION: Patients suffering from von Willebrand disease (VWD) have a variety of bleeding symptoms and require both outpatient care for treatment and, in more severe cases, hospitalization. AIM: To investigate the impact of having VWD on frequency of hospitalization compared to a control group and to evaluate whether regular replacement therapy (prophylaxis) is associated with reduction in the number of hospitalizations. METHODS: Linkage of national population-based registries was used in the Congenital Bleeding Disorders study in Sweden (CBDS)...
April 6, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29621786/relationship-between-the-von-willebrand-factor-plasma-concentration-and-ultrasonographic-doppler-findings-in-pregnancies-complicated-by-hypertensive-disorders-a-pilot-study
#4
Agata Szpera-Gozdziewicz, Tomasz Gozdziewicz, Maciej Boruczkowski, Grzegorz Dworacki, Grzegorz H Breborowicz
BACKGROUND/AIMS: Recent evidence suggests that impaired cytotrophoblast proliferation and migration are major factors responsible for the development of hypertension in pregnancy. Studies report that von Willebrand factor (vWf) is a specific endothelial damage plasma marker. The aim of this study was to evaluate the relationship between vWf maternal plasma concentration and maternal and fetal Doppler flow measurements in pregnancies complicated by hypertension. It may provide additional insight into the pathophysiology of pregnancy-related hypertension and show the potential method for disease prevention and therapy...
April 5, 2018: Gynecologic and Obstetric Investigation
https://www.readbyqxmd.com/read/29618154/low-plasma-adamts13-activity-is-associated-with-coagulopathy-endothelial-cell-damage-and-mortality-after-severe-paediatric-trauma
#5
Robert T Russell, Jenny K McDaniel, Wenjing Cao, Michelle Shroyer, Brant M Wagener, X Long Zheng, Jean-François Pittet
Decrease of plasma activity of ADAMTS13, a metalloenzyme that cleaves von Willebrand factor (VWF) and prevents adhesion and aggregation of platelets, has been reported early after onset of systemic inflammation resulting from infections and after severe trauma. Here, we determined whether trauma-induced systemic (sterile) inflammation would be associated with a reduction of plasma ADAMTS13 activity in paediatric patients and its association with disease severity and outcome. Paediatric patients ( n  = 106) with severe trauma at a level 1 paediatric trauma centre between 2014 and 2016 were prospectively enrolled...
April 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29615758/von-willebrand-factor-and-adamts13-activity-in-relation-to-risk-of-dementia-a-population-based-study
#6
Frank J Wolters, Johan Boender, Paul S de Vries, Michelle A Sonneveld, Peter J Koudstaal, Moniek P de Maat, Oscar H Franco, M Kamran Ikram, Frank W Leebeek, M Arfan Ikram
Low ADAMTS13 activity is associated with an increased risk of cardiovascular disease, which is generally attributed to its proteolytic effects on Von Willebrand factor (VWF). Cardiovascular health is an important determinant of cognitive decline, but the association of either VWF or ADAMTS13 with risk of dementia is unknown. Between 1997-2002, we measured VWF antigen and ADAMTS13 activity in 6055 participants of the population-based Rotterdam Study (mean age 69.3 years, 57.2% women). At baseline, 85 participants had dementia, and during 15 years of follow-up 821 developed dementia...
April 3, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29594657/a-predictive-value-of-von-willebrand-factor-for-early-response-to-bevacizumab-therapy-in-recurrent-glioma
#7
Andrea Pace, Chiara Mandoj, Anna Antenucci, Veronica Villani, Isabella Sperduti, Beatrice Casini, Mariantonia Carosi, Alessandra Fabi, Antonello Vidiri, Tatiana Koudriavtseva, Laura Conti
Bevacizumab (BV), a neutralizing monoclonal antibody against the vascular endothelial growth factor ligand, is recognized as a potent anti-angiogenic agent with antitumor activity. The aim of this single-center, retrospective, longitudinal study was to investigate the possible predictive value of baseline demographic, clinical and laboratory parameters for early 3-month response to BV therapy in patients with recurrent glioma. Forty-nine patients with recurrent glioma received BV at 10 mg/kg intravenously every 3 weeks alone or in association with chemotherapy were included in this study...
March 28, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29578274/desmopressin-stimulation-testing-response-to-intravenous-and-intranasal-forms
#8
M C McCormick, N Siripong, J D Cooper
INTRODUCTION: Desmopressin is commonly used to reduce bleeding in patients with mucocutaneous bleeding disorders and is available in both intravenous and intranasal forms. Given the variability in response to desmopressin at an individual level, its effectiveness should be assessed with a test dose prior to being advised for use. At this time, no trial has extensively compared the use of intranasal desmopressin to intravenous desmopressin. AIMS: To determine whether both forms of desmopressin are equally effective in yielding a positive response in laboratory assays in paediatric patients with von Willebrand disease or probable von Willebrand disease...
March 26, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29575615/spectrum-of-hemostatic-disorders-in-indian-females-presenting-with-bleeding-manifestations
#9
A Gupta, P Mishra, H P Pati, S Tyagi, M Mahapatra, T Seth, R Saxena
INTRODUCTION: Hemostatic disorders are often missed in women with bleeding particularly menorrhagia. Preexisting hemostatic disorders are now known as common risk factor for postpartum hemorrhage and prolonged bleeding in puerperium. Females with bleeding complaints constitute an important population referred to hematology clinic. Hence, we aim to evaluate the type and frequency of hemostatic disorders among females presenting with bleeding in a tertiary care hospital and a basic hemostatic laboratory...
March 25, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29573704/the-uremic-toxin-hippurate-promotes-endothelial-dysfunction-via-the-activation-of-drp1-mediated-mitochondrial-fission
#10
Mengjie Huang, Ribao Wei, Yang Wang, Tingyu Su, Ping Li, Xiangmei Chen
The accumulation of uremic toxins in chronic kidney disease (CKD) induces inflammation, oxidative stress and endothelial dysfunction, which is a key step in atherosclerosis. Accumulating evidence indicates increased mitochondrial fission is a contributing mechanism for impaired endothelial function. Hippurate, a uremic toxin, has been reported to be involved in cardiovascular diseases. Here, we assessed the endothelial toxicity of hippurate and the contribution of altered mitochondrial dynamics to hippurate-induced endothelial dysfunction...
March 16, 2018: Redox Biology
https://www.readbyqxmd.com/read/29573506/analysis-of-current-perioperative-management-with-haemate-%C3%A2-p-humate-p-%C3%A2-in-von-willebrand-disease-identifying-the-need-for-personalized-treatment
#11
H C A M Hazendonk, J M Heijdra, N C B de Jager, H C Veerman, J Boender, I van Moort, R A A Mathôt, K Meijer, B A P Laros-van Gorkom, J Eikenboom, K Fijnvandraat, F W G Leebeek, M H Cnossen
INTRODUCTION: Patients with Von Willebrand disease (VWD) are regularly treated with VWF-containing concentrates in case of acute bleeding, trauma and dental or surgical procedures. AIM: In this multicentre retrospective study, current perioperative management with a von Willebrand factor (VWF)/Factor VIII (FVIII) concentrate (Haemate® P) in patients with VWD was evaluated. PATIENTS/METHODS: Patients with VWD undergoing minor or major surgery between 2000 and 2015, requiring treatment with a VWF/FVIII concentrate (Haemate® P), were included...
March 24, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29571895/low-dose-cadmium-upregulates-the-expression-of-von-willebrand-factor-in-endothelial-cells
#12
Xia Wang, Fengyun Dong, Fufang Wang, Suhua Yan, Xiaocui Chen, Hideto Tozawa, Toshiyuki Ushijima, Carolyn M Kapron, Youichiro Wada, Ju Liu
Cadmium (Cd) is a persistent and widespread environmental pollutant of continuing worldwide concern. Previous studies have suggested that Cd exposure increases the risk of cardiovascular diseases, such as atherosclerosis and hypertension. However, the underlying mechanisms are poorly understood. In this study, we observed that low dose Cd treatment induced von Willebrand factor (vWF) expression in vascular endothelial cells in mouse lung and kidney tissues. In vitro analysis showed that 1 μM Cd specifically upregulated vWF mRNA and protein expression in human umbilical vein endothelial cells (HUVECs), indicating that Cd targets vascular endothelial cells even at relatively low concentrations...
March 20, 2018: Toxicology Letters
https://www.readbyqxmd.com/read/29568223/acquired-von-willebrand-syndrome-complicating-nephrotic-syndrome-a-case-of-a-patient-with-membranous-nephropathy
#13
Taro Sugase, Tetsu Akimoto, Takaaki Kimura, Takashi Yagisawa, Eiji Kusano, Daisuke Nagata
Acquired von Willebrand syndrome (AVWS) is a rare clinical entity presenting with heterogeneous hemorrhagic manifestations, although some subsets of patients with AVWS may be asymptomatic until they are exposed to major trauma, an invasive procedure, or surgery. We herein report one such case in a 73-year-old male patient with nephrotic syndrome with a prolonged active partial thromboplastin time. We initially did not deal with this distinct abnormal clotting profile seriously, but persistent bleeding after a retroperitoneoscopic-assisted renal biopsy that allowed us to ascribe his nephrotic syndrome to membranous nephropathy fortuitously led to the discovery of concurrent AVWS...
2018: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29565968/blood-coagulation-abnormalities-in-multibacillary-leprosy-patients
#14
Débora Santos da Silva, Lisandra Antonia Castro Teixeira, Daniela Gois Beghini, André Teixeira da Silva Ferreira, Márcia de Berredo Moreira Pinho, Patricia Sammarco Rosa, Marli Rambaldi Ribeiro, Monica Di Calafiori Freire, Mariana Andrea Hacker, José Augusto da Costa Nery, Maria Cristina Vidal Pessolani, Ana Maria Freire Tovar, Euzenir Nunes Sarno, Jonas Perales, Fernando Augusto Bozza, Danuza Esquenazi, Robson Queiroz Monteiro, Flavio Alves Lara
BACKGROUND: Leprosy is a chronic dermato-neurological disease caused by Mycobacterium leprae infection. In 2016, more than 200,000 new cases of leprosy were detected around the world, representing the most frequent cause of infectious irreversible deformities and disabilities. PRINCIPAL FINDINGS: In the present work, we demonstrate a consistent procoagulant profile on 40 reactional and non-reactional multibacillary leprosy patients. A retrospective analysis in search of signs of coagulation abnormalities among 638 leprosy patients identified 35 leprosy patients (5...
March 2018: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29564686/ofatumumab-for-acute-treatment-and-prophylaxis-of-a-patient-with-multiple-relapses-of-acquired-thrombotic-thrombocytopenic-purpura
#15
Hanny Al-Samkari, Rachael F Grace, Jean M Connors
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use...
March 21, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29562472/-mutation-analysis-of-vwf-gene-in-two-patients-with-von-willebrand-disease-by-target-sequence-capture-and-high-throughput-sequencing-technology
#16
Y Feng, S Y Mei, N Liu, R X Guo
No abstract text is available yet for this article.
March 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29553851/adjuvant-radiotherapy-does-not-affect-hemostasis
#17
Katrine Munk Begtrup, Johanne Andersen Hojbjerg, Birgitte Vrou Offersen, Anne-Mette Hvas
Cancer is associated with increased risk of venous thromboembolic disease. Venous thromboembolic disease accounts for a substantial addition to morbidity and mortality rates in cancer patients and is the second leading cause of death in cancer patients, exceeded only by the underlying cancer. Only few previous studies have investigated the influence of radiotherapy on hemostasis and whether radiotherapy in itself causes an increased risk of venous thromboembolic disease. The aim was to investigate if adjuvant radiotherapy affects hemostasis after surgery and chemotherapy in patients with breast cancer...
March 19, 2018: Platelets
https://www.readbyqxmd.com/read/29548756/ultrasound-molecular-imaging-of-atherosclerosis-using-small-peptide-targeting-ligands-against-endothelial-markers-of-inflammation-and-oxidative-stress
#18
Federico Moccetti, Craig C Weinkauf, Brian P Davidson, J Todd Belcik, Edmund R Marinelli, Evan Unger, Jonathan R Lindner
The aim of this study was to evaluate a panel of endothelium-targeted microbubble (MB) ultrasound contrast agents bearing small peptide ligands as a human-ready approach for molecular imaging of markers of high-risk atherosclerotic plaque. Small peptide ligands with established affinity for human P-selectin, VCAM-1, LOX-1 and von Willebrand factor (VWF) were conjugated to the surface of lipid-stabilized MBs. Contrast-enhanced ultrasound (CEUS) molecular imaging of the thoracic aorta was performed in wild-type and gene-targeted mice with advanced atherosclerosis (DKO)...
March 13, 2018: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/29543379/acute-coronary-syndrome-remodels-the-antiplatelet-aggregation-properties-of-high-density-lipoprotein-particle-subclasses
#19
Cédric Garcia, Nicolas Montée, Julien Faccini, Jennifer Series, Olivier Meilhac, Anne-Valérie Cantero, Pauline Le Faouder, Meyer Elbaz, Bernard Payrastre, Cécile Vindis
BACKGROUND: While high-density lipoproteins (HDL) have anti-thrombotic effects by reducing platelet activation, the relationship between HDL levels and the risk of acute coronary syndrome (ACS) is unclear since HDL particles are heterogeneous in composition and biological properties OBJECTIVE: We characterized the effects of HDL2 and HDL3 subclasses from ACS patients and non-coronary artery disease (CAD) subjects on platelet activation. METHODS: We measured platelet aggregation and ex vivo thrombus formation, analyzed signaling pathways by flow cytometry and performed a targeted lipidomics analysis on HDL subclasses RESULTS: Analysis of human platelet aggregation in suspension, adhesion on von Willebrand factor and thrombus formation on collagen under arterial shear demonstrated that HDL2 subclass from ACS patients had a higher antiplatelet potency compared to ACS HDL3 particles and HDL subclasses from non-CAD subjects...
March 15, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29523490/thrombotic-microangiopathy-in-a-very-young-infant-with-mitral-valvuloplasty
#20
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
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